The modulation of the HDL receptor scavenger receptor B1 (SRB1) was evaluated in skin fibroblasts isolated from patients with Rett syndrome (RTT), a genetic form of infantile autism. Patients showed an altered plasma lipid profile, while their skin fibroblasts showed a dramatic reduction in SRB1 (immunogold, Western blot and immunohistochemistry). The decreased SRB1 levels were demonstrated to be the consequence of its binding with 4-hydroxy-2-nonenal (4HNE), a product of lipid peroxidation, and its increased ubiquitination. Our findings show for the first time a loss of SRB1 in RTT cells and its relationship with a chronic oxidative stress status. © 2013 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.

Scavenger receptor B1 post-translational modifications in Rett syndrome

STICOZZI, Claudia
Primo
;
Pecorelli A.;CERVELLATI, Franco;VALACCHI, Giuseppe
Ultimo
2013

Abstract

The modulation of the HDL receptor scavenger receptor B1 (SRB1) was evaluated in skin fibroblasts isolated from patients with Rett syndrome (RTT), a genetic form of infantile autism. Patients showed an altered plasma lipid profile, while their skin fibroblasts showed a dramatic reduction in SRB1 (immunogold, Western blot and immunohistochemistry). The decreased SRB1 levels were demonstrated to be the consequence of its binding with 4-hydroxy-2-nonenal (4HNE), a product of lipid peroxidation, and its increased ubiquitination. Our findings show for the first time a loss of SRB1 in RTT cells and its relationship with a chronic oxidative stress status. © 2013 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
2013
Sticozzi, Claudia; Belmonte, G.; Pecorelli, A.; Cervellati, Franco; Leoncini, S.; Signorini, C.; Ciccoli, L.; De Felice, C; Hayek, J; Valacchi, Giuseppe
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/1814503
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